The medications listed under this medical policy may be subject to the Redirection of Care Program. Some contracts may require medications to be provided in a non-hospital based setting. For more information, please visit https://www.bcbswy.com/providers/site-of-care/.

Background

Evkeeza (evinacumab-dgnb) is a recombinant human monoclonal antibody that binds to and inhibits ANGPTL3 (angiopoietin-like 3). ANGPTL3 is a member of the angiopoietin-like protein family that is expressed primarily in the liver and plays a role in the regulation of lipid metabolism by inhibiting lipoprotein lipase (LPL) and endothelial lipase (EL). Inhibition of ANGPTL3 leads to reduction in LDL-C, HDL-C, and triglycerides. Evkeeza reduces LDL-C independent of the presence of LDL receptor by promoting very low-density lipoprotein (VLDL) processing and clearance upstream of LDL formation (1).

Regulatory Status

FDA-approved indication: Evkeeza is an ANGPTL3 (angiopoietin-like 3) inhibitor indicated as an adjunct to diet and exercise and other low-density lipoprotein-cholesterol (LDL-C) lowering therapies to reduce LDL-C in adults and pediatric patients, aged 1 year and older, with homozygous familial hypercholesterolemia (HoFH) (1).

Serious hypersensitivity reactions have occurred with Evkeeza. If signs or symptoms of serious hypersensitivity reactions occur, Evkeeza should be discontinued, and the patient should be treated according to the standard-of-care and monitored until signs and symptoms resolve. Evkeeza is contraindicated in patients with a history of serious hypersensitivity reaction to evinacumab-dgnb (1).

Evkeeza may cause fetal harm when administered to pregnant patients. Consider obtaining a pregnancy test prior to initiating treatment with Evkeeza. Patients who may become pregnant should be advised to use effective contraception during treatment with Evkeeza and for at least 5 months following the last dose of Evkeeza (1).

Evkeeza was studied in combination with other adjunct therapies, including proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors and Juxtapid (lomitapide) in particular. Patients were stable on these therapies for at least 8 and 12 weeks, respectively before adding Evkeeza to their regimen. Evkeeza may be considered in patients that have not met their lipid goal on other therapies (2).

The safety and effectiveness of Evkeeza in patients less than 1 year of age have not been established (1).

Evkeeza may be considered medically necessary if the conditions indicated below are met.

Evkeeza may be considered investigational for all other indications.

Prior-Approval Requirements

Age        1 year of age or older

Diagnosis

Patient must have the following:

Homozygous familial hypercholesterolemia (HoFH)

AND ALL of the following:

1.       Documented confirmation of diagnosis by LDL-R DNA Sequencing Test or APOB (hypercholesterolemia) Mutation Analysis

2. Genetic confirmation of two mutant alleles at the LDLR, Apo-B, PCSK9, ARH adaptor protein 1/LDLRAP1 gene locus

3. Patient has a history of an untreated total cholesterol level of greater than 500 mg/dL AND ONE of the following:

a. Presence of cutaneous or tendinous xanthomas before the age of 10 years

b. Untreated total cholesterol level of more than 250 mg/dL in both parents

4. Treated LDL-C ≥ 300 mg/dL

5. Patient has had an inadequate treatment response on at least TWO lipid-lowering therapies (e.g., statins, ezetimibe) at the maximum tolerated dose, or intolerance due to persistent skeletal muscle related symptoms

6. 10 years of age or older only: Patient has ONE of the following: a. LDL remains > 70 mg/dL after at least 8 weeks of continuous treatment with a PCSK9 inhibitor b. An intolerance or contraindication to a PCSK9 inhibitor

7. Used in combination with diet and exercise

8. Used in combination with other lipid-lowering treatments (e.g., statins, ezetimibe)

9. Prescriber agrees to monitor for hypersensitivity reactions to Evkeeza

10. Females of reproductive potential only: patient will be advised to use effective contraception during treatment with Evkeeza and for 5 months after the last dose

Prior – Approval Renewal Requirements

Age        1 year of age or older

Diagnosis

Patient must have the following:

Homozygous familial hypercholesterolemia (HoFH)

AND ALL of the following:

1. Reduction in LDL-C from baseline

2. Used in combination with diet and exercise

3. Used in combination with other lipid-lowering treatments (e.g., statins, ezetimibe)

4. Prescriber agrees to monitor for hypersensitivity reactions to Evkeeza

5. Females of reproductive potential only: patient will be advised to use effective contraception during treatment with Evkeeza and for 5 months after the last dose

Pre - PA Allowance

None

Prior - Approval Limits

Duration 12 months

Prior – Approval Renewal Limits

Same as above